64 Conical teeth? Think outside the box!

Abstract Background Ectodermal dysplasias (ED) are inherited disorders involving congenital abnormalities of different ectodermal structures, the most prominent presentation being adontia/hypodontia In this article, we present a brief overview of several pediatric cases of ED with a short review of the odontological manifestations. 2. Case reports We report several cases of children consulting in the pediatric department for abnormal teeth, with or without systemic manifestations. The clinical examination of these patients revealed insufficient and abnormal dentition, with rare beveled teeth and thin and rare hair, as well as eyelashes and eyebrows. Also, parents reported episodes of hyperthermia without sweating from an early age. Dental radiography confirmed the diagnosis of ectodermal dysplasia. 3. Discussion Ectodermal dysplasias (EDs) are a group of various inherited disorders involving abnormal congenital development of at least two ectodermal structures (hair, nails, teeth, and sweat glands). The management of these rare disabling conditions is still mainly symptomatic. The traditional removable prosthesis can be an option to replace missing teeth. However, implants provide the best long-term results and prognosis. In particular, dental implants are commonly used in oral reconstruction of ED patients, but long-term data on bone augmentation and bone resorption, aesthetic outcomes, and implant success are needed. 4. Conclusion EDs are a myriad of heterogeneous conditions encompassing several inherited embryopathies affecting teeth and other ectoderm-derived structures in utero. Diagnosis is essentially clinical, confirmed by radiology and genetics which can specify the causal mutation; treatment is mainly conservative.


Background
Spinal pain is a frequent symptom whose aetiology can be extremely varied. It is not only the prerogative of adults; it is frequent in children and particularly in adolescents. Inflammatory spinal pain is a warning sign that should prompt further investigations to rule out an underlying pathology such as infectious spondylodiscitis, malignant pathology, ankylosing spondylitis or SAPHO syndrome. In this case, we report on a 15-year-old child with uncommon inflammatory spinal pain that caused diagnostic difficulties. Observation The child B.A., aged 15 years, with no previous history, consulted for inflammatory back pain that had been evolving for >3 months in a context of apyrexia and conservation of the general state, associated with pain of the anterior thoracic wall. The clinical examination revealed an exaggerated dorsal kyphosis, a lumbar spinal syndrome. The rest of the joint examination was without abnormalities and the somatic examination revealed a retention acne. Biologically, there was no inflammatory syndrom, brucella serology and tubeculin TST were negative. An X-ray and a CT scan of the dorsal-lumbar spine showed multiple bone erosions of the vertebral plates and a fracture of the T6 and T7 vertebrae with global disc pinching, without recession of the posterior wall or damage to the soft tissues and with exaggeration of the dorsal cyphosis at this level. The spinal MRI revealed a spondylodiscitis with hypo T1 hyper T2 signal at the T6-T7 level without any collection. An osteolytic lesion of the sternal manubrium lateralized to the left with blurred contours was objectified by the CT scan of the anterior chest wall. Bone scans revealed intense hyper fixation foci at the T6-T7 level and the sternal manubrium. MRI of the sacroiliac joints was normal. A scan-guided biopsy of the T6-T7 level did not show any specific lesion. In view of the inflammatory spinal pain, the anterior chest wall pain, the negativity of the biological tests and the imaging data, the diagnosis of incomplete SAPHO syndrome was retained after having ruled out other etiologies. SAPHO syndrome is a rare entity that refers to the association of a heterogeneous set of cutaneous and osteoarticular manifestations with a common denominator of an aseptic inflammatory process. It remains a diagnosis of elimination.

Conclusion
Spinal pain in children can be a symptom of multiple etiologies and sometimes poses a diagnostic problem. This is a particular observation and deserves to be reported because unusual clinical forms of SAPHO syndrome exist and should not be ignored.

Background
The term ''dentinogenesis imperfecta'' includes dental anomalies quite similar to hereditary opalescent dentin and osteogenesis imperfecta. We present dental findings in a boy with osteogenesis imperfecta type IV Case Presentation A young boy was referred to the pediatric department for management of vertebral compression and a recurrent femoral fracture without obvious trauma. The appearance of his teeth with the overall assessment of this patient reveals dentinogenesis imperfecta compatible with osteogenesis imperfecta type IV, associated with major vertebral involvement Discussion Osteogenesis imperfecta (OI), also known as also known as « brittle bone disease » is a heterogeneous group of rare genetic diseases involving connective tissue. A reduction in the amount of normal collagen leads to Type I, while qualitative and quantitative abnormalities in collagen synthesis lead to Types II, III and IV of OI. This type IV is subdivided into groups A and B: the group B depicting the characteristic dentinogenesis imperfecta like in our patient. Conclusion Dentinogenesis imperfecta is a major sign of osteogenesis imperfecta. Skeletal phenotype, scleral colour and deafness complete the picture allowing the final classification of this inherited disorder The peculiar bone lesions with normal dental aspects are highly of the extreme rare osteogenesis imperfecta type IV. Background Ectodermal dysplasias (ED) are inherited disorders involving congenital abnormalities of different ectodermal structures, the most prominent presentation being adontia/hypodontia In this article, we present a brief overview of several pediatric cases of ED with a short review of the odontological manifestations.

Case reports
We report several cases of children consulting in the pediatric department for abnormal teeth, with or without systemic manifestations. ii24

E POSTERS
The clinical examination of these patients revealed insufficient and abnormal dentition, with rare beveled teeth and thin and rare hair, as well as eyelashes and eyebrows. Also, parents reported episodes of hyperthermia without sweating from an early age. Dental radiography confirmed the diagnosis of ectodermal dysplasia.

Discussion
Ectodermal dysplasias (EDs) are a group of various inherited disorders involving abnormal congenital development of at least two ectodermal structures (hair, nails, teeth, and sweat glands). The management of these rare disabling conditions is still mainly symptomatic. The traditional removable prosthesis can be an option to replace missing teeth. However, implants provide the best long-term results and prognosis. In particular, dental implants are commonly used in oral reconstruction of ED patients, but long-term data on bone augmentation and bone resorption, aesthetic outcomes, and implant success are needed.

Conclusion
EDs are a myriad of heterogeneous conditions encompassing several inherited embryopathies affecting teeth and other ectoderm-derived structures in utero. Diagnosis is essentially clinical, confirmed by radiology and genetics which can specify the causal mutation; treatment is mainly conservative.

Background
Pediatric Rheumatology is currently gaining importance across Africa. Initiatives such as the creation of the paediatric society of the African league against rheumatism (PAFLAR) proved the efforts made to bridge the insufficiency. In the same line, training in pediatric rheumatology remains the main challenge of this orphan subspecialty. For this purpose, a post-graduate certification in Pediatric Rheumatology was created in 2021 in the Faculty of Medicine of Tunis-Tunis-El Manar University. It is the first online certification dedicated to French speakers in Africa. The certification is entitled ''musculoskeletal diseases of children and adolescents''.

Objective
To assess the learners' perceptions and views of this certification.

Methods
We established an online survey targeting the participants. The questionnaire was divided into three main sections: the first section examined the participant characteristics: their specialty, the use of the Moodle platform of the Virtual University of Tunis (UVT), their previous participation in an online post-graduation course, etc.). The second section focused on platform acceptability. The third and final section evaluated the content of the course. The Likert scale was used in most questions.

Results
Fifteen participants responded to the survey. Among them, one is specialized in family medicine, 1 in orthopedics, 5 in pediatrics, and 8 in rheumatology. Fifty-three percent have never used the platform of the virtual university of Tunis (UVT) before, and 26.7% have never attended an online course. Most participants found the platform easy to access and easy to use with a mean Likert scale of 2.2 and 2.13, respectively. In the third section of the questionnaire, the course met the expectations of the candidates with a mean Likert scale of 2.1. Most of them found the post-test useful to their learning experience, and only two found it to be the opposite. This can be explained by the pressure that it may generate. Eighty-six percent of the candidates had an interactive experience with the educator and enjoyed the interactions and the discussions between the participants. Most participants were pleased with the seminar organization and with the number of h devoted to each seminar (mean Likert Scale ¼ 2.1).
Eighty-six percent of the participants have found that the course improved their medical practice and 93.3% confessed that they were satisfied with the online nature of the certification and did not wish it to be physical.

Conclusion
Overall, the post-graduate ''musculoskeletal diseases of children and adolescents'' course was beneficial and enriching/rewarding for the participants.
Developing this training program helps to promote pediatric rheumatology in Africa and to provide children with rheumatic diseases access to healthcare.

Introduction
Optic neuritis (ON) are rare in children. They are due to inflammation of the optic nerve which results in a sudden drop in visual acuity, or amputation of the visual field. They are often secondary to an infectious disease or vaccination, but they can be part of autoinflammatory, demyelinating or systemic diseases. The features of optic neuritis in children are different from adults as regards high rate of bilateral involvement, poor visual acuity, and papillitis.

Aim
Our aim is to describe the clinical and therapeutic outcomes of patients diagnosed with optic neuritis in the general pediatrics department at the CHU of Bab el Oued in Algiers.

Methods
It is a retrospective, descriptive study of patients who were treated for optic neuritis in our hospital over a period of three years (January 2018 to April 2021).

Results
There were 7 cases, 4 girls and 3 boys with F: M of 1.3:1, mean age of 10 years and age range of 6-13 years. The first manifestation was a decrease in visual acuity in all cases, 12 eyes affected, with bilateral involvement in 5 patients. Visual acuity decrease was severe < 3/10 in 80% of cases. The visual field was pathological in 2 cases (narrow, central scotoma). All patients had headaches. Retrobulbar involvement was confirmed by visual evoked potentials in all patients, they showed demyelinating involvement. Three patients presented papillitis. The diagnostic work-up included cerebral imaging which showed old nodular abnormalities of signal of the subcortical white matter in one patient. CSF analysis revealed anti-MOG antibodies in only one case. The infection investigations were negative in all cases except positive COVID 19 serology in one case The diagnosis retained in our patients were 2 cases of Behcet's disease, 1 monophasic neuritis, 1 optic neuritis with anti-MOG, 1 CRION, 1 case linked to COVID 19 in the absence of another plausible cause. In one case, no diagnosis was retained. Seventy percent of the patients benefited from treatment with corticosteroids pulse, only 2 did not receive treatment, the shortterm evolution was favorable in 5 patients with a total recovery of visual acuity, one patient has evolved into atrophy of the left optic nerve and kept visual acuity low to 1/10 of the left eye. The medium-term evolution was marked by recurrence in 3 patients, 5 episodes in the 2 patients with Behcet's disease and 8 episodes in CRION which required the use of immunosuppressive treatment and biotherapy for Behcet's disease and plasmapheresis for CRION.

Conclusion
Despite the fact that our small series of patients is not representative, we found similar characteristics described in children in other studies. The average onset age was 10 years, with a female predominance, the severe decrease in visual acuity with frequent bilateral involvement. The diagnosis of ON is clinical and must be considered in any sudden drop in visual acuity. The diagnosis of the underlying pathology is often difficult but it's important in order to propose an adequate specific treatment. The treatment of children's ON is controversial and usually extrapolated from adults. Most practitioners use corticosteroids pulse.
The visual prognosis seems good in children, particularly in the case of bilateral involvement, but depends essentially on the aetiology.